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1例抗表皮下脓疱性类天疱疮伴广泛皮肤糜烂,酷似寻常型天疱疮。

A case of antiepiligrin cicatricial pemphigoid with extensive cutaneous erosions mimicking pemphigus vulgaris.

作者信息

Suvirya Swastika, Pathania Sucheta, Malhotra Kiran Preet, Verma Parul

机构信息

Department of Dermatology, King George's Medical University, Lucknow, Uttar Pradesh, India.

Department of Pathology, Dr. Ram Mahohar Lohia Institute of Medical Sciences, Lucknow, India.

出版信息

BMJ Case Rep. 2018 Jun 21;2018:bcr-2018-225446. doi: 10.1136/bcr-2018-225446.

Abstract

Cicatricial pemphigoid (CP) is a chronic, autoimmune, subepidermal blistering disease with predominant mucosal involvement. In this article, we report a young patient with mucosal and extensive cutaneous involvement in the form of large erosions mimicking those of pemphigus vulgaris thus leading to diagnostic dilemma. We were unable to find any other previous reports with such extensive cutaneous erosions mimicking those of pemphigus vulgaris. Laminin 5 was the antigen found on knockout substrate testing. Antiepiligrin CP is a distinct subtype of CP with antibodies against laminin 5. This subtype is mostly associated with malignancy but no underlying malignancy was found in our case. Present report also highlights the importance of knockout substrate testing when immunoblot is not available.

摘要

瘢痕性类天疱疮(CP)是一种慢性自身免疫性表皮下大疱性疾病,主要累及黏膜。在本文中,我们报告了一名年轻患者,其黏膜和广泛皮肤受累,表现为类似寻常型天疱疮的大面积糜烂,从而导致诊断困境。我们未找到之前有类似广泛皮肤糜烂且类似寻常型天疱疮的其他报道。层粘连蛋白5是在免疫印迹试验中发现的抗原。抗表皮下大疱性类天疱疮抗原的CP是CP的一种独特亚型,具有抗层粘连蛋白5的抗体。该亚型大多与恶性肿瘤相关,但我们的病例中未发现潜在恶性肿瘤。本报告还强调了在无法进行免疫印迹时免疫印迹试验的重要性。

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